This Is MS Multiple Sclerosis Community: Knowledge & Support

Welcome to the world's leading forum on Multiple Sclerosis research, support, and knowledge. For over 10 years, This is MS has provided an unbiased community dedicated to Multiple Sclerosis patients, caregivers, and affected loved ones.

Hello Everyone,My 1000th post on TiMS is to highlight Prof Zamboni's papers. If you are new to TiMS these are crucial to your understanding of CCSVI. If you have been posting on the CCSVI forum for some time and have not read these papers in in last 12 months, please refresh your memory.Kind regards,Mark

AbstractObjectives: To evaluate the role of the omohyoid muscle anatomic variants as a possible reversible cause of internaljugular vein extrinsic compression.

Method: We describe a chronic cerebro-spinal venous insufficiency patient, who presented a omohyoid muscle entrapmentof the internal jugular vein, confirmed by both magnetic resonance venography and ultrasound investigation.A omohyoid muscle surgical transection together with a patch angioplasty was performed.

Results: The surgical procedure led to both IJV flow restoration and neurological improvement.

Conclusions: The omohyoid muscle compression on the internal jugular vein seems to be a possible cause of venousobstruction, but several anatomical and patho-physiological aspects need further investigations. Such picture might causeballoon venous angioplasty inefficacy and needs to be preoperatively considered.

Two things were treated: a muscle was snipped that was compressing the jugular, and the jugular itself underwent a patch angioplasty, which is when the jugular is cut lengthwise and a patch of additional vein is sewn in to make the jugular bigger.

Since both a patch angioplasty and a muscle transection occurred, then we don't know which of the two procedures gets the credit for the neurological and blood flow improvements, but there were improvements!

Chronic cerebrospinal venous insufficiency (CCSVI) is a newly described vascular entity. It is characterized by restricted venous outflow from the brain and spinal cord through the internal jugular (IJV) and/or azygos (AZ) veins (Figure 1), or sometimes, through the external vertebral plexuses. (1-5)

Left) Catheter venography of the internal jugular vein in healthy control; Right) Stenosis (arrows) and collateral circles activated in a CCSVI case, both studied by the means of catheter venography.

Clinical Vignette: CCSVI in multiple sclerosis patients

A 41 year old woman, diagnosed with relapsing-remitting multiple sclerosis, came to our office with the following history. She underwent color Doppler and phlebographic evaluation 3 years ago in an interventional center. The pre-assessment disability score (EDSS), (normal range0 to 10), was 3. The investigation revealed a CCSVI related left IJV stenosis, associated with a color Doppler detectable blocked outflow and linked to an M-mode evident fixed valve apparatus. (14) A PTA was performed leading to an effective IJV widening at 6 month follow up. At the same time the EDSS decreased to 2 and the patient described a clinical improvement of the pre-assessment reported paresthesia and fatigue.

At 1 year follow up the EDSS was 3 again and the patient reported recurrent fatigue that she had been experiencing for several months. Further investigation revealed a CCSVI related left IJV tandem obstruction. At mid cervical level B-mode color Doppler demonstrated an IJV obstruction mirrored by an MRV imaging. In both cases a pencil tip pencil sign was demonstrated (Figure 2). The color Doppler assessment highlighted a left IJV dynamic flow obstacle caused by an extrinsic compression (Figure 2), that could be relieved by yawning.

Top: the arrow in the MRV indicates an obstruction of the left IJV due to external compression. Bottom: left, particular of the tip pencil sign mirrored on the right by the correspondent high-resolution B-mode imaging of the left IJV.

At the caudal level a color Doppler detectable blocked outflow was demonstrated. The flow abnormality was linked to a M-mode evident fixed valve leaflet.

Considering a further PTA pointless, an open surgical access was placed. The macroscopic evidence of the extrinsic stricture, caused by a fibrotic and short OM intermediate tendon on the IJV, lead to the surgical transection of the two muscular bellies. In addition, endophlebectomy of the terminal IJV permitted the removal of a fibrotic septum. The procedure was completed by patch angioplasty by using an autologous great saphenous vein. The patient tolerated the procedure well and neither major nor minor complications were reported.

At 2 years follow up after the surgical operation, the color Doppler assessment detected a persistent and monodirectional physiologic IJV flow, which improved considerably from being absent pre-operatively to the post-procedural 300 ml/min. The a EDSS value remained 1 and the patient did not report any other multiple sclerosis like relapses.

Pathophysiology and diagnosis of CCSVI

Occlusion of the extracranial venous pathways can take various forms, including the presence of intraluminal septa, membranes, and immobile valves, as well as segmentary hypoplasia of the veins. (1-5) Similar intraluminal obstructions and hypoplasia\agenesis have been documented for other malformations of the venous tree. These have been documented with a combination of techniques (intra and extravascular ultrasounds, catheter and MR venography). (6-7)

It is difficult to accurately non-invasively screen for CCSVI, due to the high operator-dependency of echo color Doppler (ECD) sonography techniques. For this reason the reported prevalence of CCSVI measured by the means of ECD is highly heterogeneous in the literature. However this diagnosis may be important to make. Specifically, CCSVI may be associated with neurodegenerative disorders such as multiple sclerosis (MS) – something that has generated considerable scientific controversy, (8-12) A meta-analyses revealed that CCSVI was found mainly in multiple sclerosis (MS) patients (OR 4, 95% CI 1-11), but was also possibly associated to other neurodegenerative diseases. Complicating matter further, it has also been found in healthy controls. (13)

CCSVI and brain pathophysiology

CCSVI appears to strongly influence two pivotal aspects of brain pathophysiology: parenchymal perfusion and cerebrospinal fluid (CSF) flow. CCSVI affects the venous return and MRI studies have shown that it is strongly associated with hypoperfusion of brain parenchyma, (Figure 3). Interestingly, this has consistently been described in multiple sclerosis patients yet the autoimmune theory of the disease cannot explain it. (15-16)

Furthermore, CCSVI is linked to altered CSF dynamics (17-18), suggesting that intracranial hydrodynamic regulatory mechanism may be impaired in individuals who exhibit CCSVI. CSF is filtered at the capillary level and flows in a closed circuit. The end terminal is represented by the cerebral venous system, which in CCSVI exhibits a hampered outflow. Interestingly, PTA of the jugular and azygous veins improves both CSF flow and velocity as blindly assessed in the brain by the means of 3TMR. (19) Such an effect on CSF dynamics seems particularly relevant because in neurodegenerative disorders CSF reduced flow is inversely related to accumulation of T2 lesions at MRI. (20)

CCSVI and Interventional procedures

PTA of the jugulars and azygous system is considered a safe procedures, whereas the risk is increased if a stent is used.(21-23) Clinical and QoL improvements have been reported in a number of prospective and case control studies following interventional procedures, (24-30) and a double blinded randomized trial is currently enrolling. (31) Read more about venoplasty for multiple sclerosis.

Thanks for this. It is nice to know that, when some physicians are willing to allow their contemporaries to treat, PTA may be followed by other successes.

Too bad that in all the hullabaloo over who has the biggest dick, something that was tried with some success in Canada, as an alternative to fetal cells, is now co-opted by the FDA and will be considered a drug, to prop up a bloated industry.

As usual, human life is secondary to money. Well, I guess any professional killer could have told me that.

Who is online

This site does not offer, or claim to offer, medical, legal, or professional advice.
All treatment decisions should always be made with the full knowledge of your physicians.
This is MS does not create, endorse, or republish any content.
All postings are the responsibility of the poster. All logos and trademarks in this site are property of their respective owners. All users must respect our rules for intellectual property rights.